Spina Bifida

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Nivedita.P.Kashyap
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Spina Bifida is a latin word for split spine.
Most common group of birth defects called
neural tube defects (NTD).
The neural tube develops into the brain and
spinal cord.
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Definition :
It is a congenital abnormality with developmental
defect in the spinal column with incomplete
closure of vertebral canal due to failure in fusion
of vertebral arches ± protrusion and dysplasia of
the spinal cord or its membranes.
Spina Bifida is a spinal defect usually diagnosed
at birth by the presence of an external sac on the
infants back.ti tube
birth defect resulting from the incomplete
closure of the embryonic neural tube
the abnormal portion of the spinal cord sticks
out through the opening in the bones
vertebrae overlying the open portion of the
spinal cord remains unfused and open
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It’s a defect of the spine in a developing fetus, it
affects the brain,spinal cord,and it’s muscles
surrounding it, and resulting in loss of movement
and or sensation to the legs and feet as well as
bowel movement and bladder dysfunction.
When does Spina Bifida occur?
 Occurs between days 24 and 28 of gestation.
 This disorder arises from abnormalities occuring
during three major concurrent migrations of cell
groups during embryogenesis which include
those of the neural tube, the notochord, and the
mesenchymal tissue.
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The notochord is a column of cells that run up
the length of the embryo between the ectoderm
and endoderm. It plays an important role in
inducing formation of the spinal cord and spinal
column.
The neural tube is the precursor of the brain and
spinal cord. At this stage the ectodermal cells in
the midline differentiate into neuroectoderm to
form the neural plate. The neural plate elevates
to form neural folds that then meet or close at
midline to form the neural tube.
Normal embryological
development
Neural plate
development -18th day
Cranial closure 24th
day (upper spine)
Caudal closure 26th
day (lower spine)
Neural Tube defects may result from:
 Combination of environmental and genetic causes
 Teratogens
 Nutritional deficiencies - notably, folic acid
deficiency
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Women with certain chronic health problems such
as diabetes and seizure disorders have an
increased risk of having a baby with spina bifida
especially if taking anticonvulscent medications.
1- Closed : Occulta = hidden
2- Opened: Cystica = Manifesta
Forms of spina bifida
 Occulta
 Meningocele
 Myelomeningocele
Occulta :
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Mildest form of spina bifida
The outer part of some of the vertebrae are not
completely closed
gap in one or more of the vertebre of the spine
The split in the vertebrae is so small that the spinal
cord does not protrude
The condition is asymptomatic
Bony abnormality seen by X – ray
Nerves may be involved when associated with hairy
patch or other skin changes
dimple, hairy patch, dark spot or swelling over
affected area
the spine
dimple, hairy patch, dark spot or swelling over affected area
spinal cords and nerves usually normal
no treatment needed
Cystica:
1- Meningeocele:
Rarest form a cyst consisting of membranes
surrounding the spinal cord pokes through
the open part of the spine.
Can be removed surgically allowing for
normal development.
sac contains membranes that
protect spinal cord, but not spinal nerves
Myelomeningeocele:
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Sac contains neural elements that protrude through the spinal
defects
The overlying skin is thin & leaks of spinal fluid
Secondary infection is common , Neurological and Orthopaedic
problem are present.
Hydrocephalus(arnold- chiari malformation)
Clinical Picture: It will differ according to the level of lesion (most
common site is lumbosacral region)
 Flaccid paralysis, muscle weakness, wasting, decreased or absent
tendon reflexes
 Decreased or absent extroceptive & proprioceptive sensation
 Rectal & bladder incontinence, Hydrocephalus, Sever vasomotor
changes
 Paralytic or congenital deformities as in club foot , pes cavus.
 Pressure ulceration due to poor sensation, Osteoporosis , soft
tissue contracture
 Physical emotion& mental delay
Prognosis:
With successful closure of simple meningeocele prognosis is good
Myelomeningeocele die from infection, if survive after proper closure ---------- stationary disability
Lipomeningocele
(lipo = fat)
lipoma or fatty tumor located over the lumbosacral
spine. Associated with bowel & bladder
dysfunction
• - couples who already had an affected baby
have an increased risk of having another
affected baby
• - women who are obese, have poorly
controlled diabetes, or who are treated with
certain anti-seizure meds have a higher risk
• - more common in Hispanics and Caucasions
• Folic Acid -- 70% NTDs can be prevented
• multivitamin of 400 micrograms of folic acid
every day
• every day foods: grain products, fortified
foods, leafy-green vegetables, dried beans,
oranges, orange juice
Amniocentesis
AFP - indication of abnormal leakage
Blood
test
Maternal blood samples of AFP
Ultrasonography
For locating back lesion vs. cranial signs
Physiological changes below the level of the
lesion generally include:
abnormal nerve conduction, resulting in:
somatosensory losses
motor paralysis, including loss of bowel and bladder
control
Anatomical changes below the level of lesion:
musculoskeletal deformities (scoliosis)
 joint and extremity deformities (joint contractures, club foot, hip
subluxations, diminished growth of non-weight bearing limbs)
 osteoporosis
 abnormal or damaged nerve tissue
Anatomical changes associated with a cervical lesion:
An enlarged head caused by hydrocephalus
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One way that Spina Bifida is being treated is
by operating on the fetus while still in the
womb. This procedure is done as if fetus is
being delivered via cesarean section.
Another way is that it is usually treated
surgically between 24 to48 hours after birth.
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Impairments associated with spina bifida:
Physiological changes occur below level of lesion : Abnormal NCV
Lead to changes in muscle tone
 Anatomical changes below level of lesion:
 Musculoskeletal deformities(Scoliosis), osteoporosis
 Joint&extermity deformities, damaged nerve tissue
 Diminished growth of non Wt bearing joints
Complication & team work:
1- Mass& Hydrocephalus…………….Neurosuergon
2- Movement disorders, Deformity……………PT
3- Bowel& Bladder Disturbance…………..Urologist
4- Social& Psychological problem………………Psychiatrist& Social worker
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PT EXAMINATION:
1. Inspection:
 Tuft of hair, subcutaneous lipoma ordimple may be observed in
occulta
 Localised sac in cystic type, increased head size,
 Deformity of Lower limb as flexion: Abd & int rotation of hip, flexion
or hyper extension knee, equinous or calcenous deformity of foot,
scoliosis or lordosis of spine
 Skin ulceration and soft tissue injury due to pressure
2. Palpation:
Bony defect, subcutaneous lipoma, Loss of sensation and muscle bulk
3. Measurement:
a- Tape measurement : Head L.L (long& round)
b- Geniometer test ROM
4. Muscle Testing
5. Functional Test (UP & Low Limb)
1- Upper Limb Exercises: Children with spina bifida needs to
compansate weakness in their legs and trunk : Strong arms
assist for ADL such as:
 Helping in sitting without trunk stability
 Using hand-operated mobility aids: wheel chair, crutches
 Periodically rise from chair to relive pressure symptoms on
skin
 Transferring from seat to toilet, Standing up from chair
 Extending the arms to lift the seat off the floor
 Press- up with pillows under the knees and feet
2- Poor Sitting balance:
Children with spina bifida with sever high level
have a poor sitting balance due to :
Lack of dynamic stability(weak trunk muscles)
Weak trunk muscles, Paralysed leg to
counterbalance movement of upper limb
Lack of sensation from buttocks , lower limb( no
proprioception, feedback on base of balance from
Lower limb)
Large head (hydrocephalus)
How to overcome Poor Sitting balance:
 Head control Exercises
 Strengthening exercises of back muscles and
balance Exercises (Rolling, getting from prone
to sit)
 Special seats to provide adequate support
 Regular relive the pressure is necessary
P.T Modalities:
 Extensive PT program should be applied in cases
of partial paralysis
 Electrotherapy to relive pain and induce relaxation
 Passive movement and passive stretching to
maintain available ROM prevent soft tissue
contracture
 Active exercises to prevent muscle imbalance
 Hyderotherapy when skin is intact to improve ROM
 Gait training by using Braces
Bladder management:
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Renal disease is responsible for all cases of death in spina
bifida, So bladder care is vital for spina bifida children
Aims: 1- prevent infection
2- establish a
satisfactory method of emptying of bladder
A Patient with spina bifida may have one of the
following:
1- Automatic Bladder: is found in children
with cyst above T10-11 pressing on cord lead to
hyper reflexia of all muscle below level of injury.
The muscle will contract in response to certain
degree f filling that lead to open the sphincter
Empty the bladder should be performed every
one hour and the baby will remain dry.
2- Autonomous bladder(non- reflex): Most
common in spina bifida patient
Atonic bladder, No reflex action, used
suprapubic manual pressure to empty bladder
every hour should be carried out by parents to
keep the child dry until be mature enough to do it
by himself.
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Bowel management:
Bowel training performed when the patient is on
full diet
 Mild aperient in the evening
 Glycerin suppository in the morning followed 15
minutes later by digital
 evacuation
 Correct diet and fluids
 Evacuation should be conducted by patrents till
child be mature enough
 Sensation:
 Children will lack sense of pain temperature touch
pressure.
 They cannot protect themselves from hot water and
burn.
 They have insensitive feet
 Care should be taken to Ankle foot orthosis from
pressure
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Proprioception:
Lack of proprioceptive feedback from muscles lead
to lack of tactile kinetic feed back
 They rely on visual input only to develop sense of body
awareness difficulty in maintain balance and upright
sitting
Orthosis:
Ankle Foot Orthosis with or without external aids used
when
lower lumber level of lesion, power 5 of quadriceps , 4 of
medial hamstring muscles
early walking is better than wheel chair in children with
high level of lesion in spina bifida
Two orthosis are used to maintain reciprocal gait pattern
and enabling standing
Reciprocating gait orthosis and hip guidance orthosis
Prerequisites of fitting orthosis:
1- No more than 20 degree of hip flexion
2- knee and foot can be rendered in plant grade position
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